General description
This gene encodes the alpha subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I).
Immunogen
Synthetic peptide directed towards the C terminal region of human HEXA
Physical form
Supplied at 0.5 mg/ml in phosphate-buffered saline, 0.09% sodium azide
Other Notes
Synthetic peptide located within the following region: WKDFYIVEPLAFEGTPEQKALVIGGEACMWGEYVDNTNLVPRLWPRAGAV
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
| biological source | rabbit |
| conjugate | unconjugated |
| antibody form | affinity isolated antibody |
| antibody product type | primary antibodies |
| clone | polyclonal |
| form | buffered aqueous solution |
| mol wt | 48 kDa |
| species reactivity (predicted by homology) | sheep, bovine, guinea pig, pig, horse, human, canine, rabbit, mouse |
| concentration | 0.5 mg/mL |
| technique(s) | immunohistochemistry (formalin-fixed, paraffin-embedded sections): 4-8 μg/mL,western blot: 1 μg/mL |
| NCBI accession no. | NM_000520 |
| shipped in | wet ice |
| storage temp. | −20°C |
| target post-translational modification | unmodified |
| Gene Information | human ... HEXA(57396) |
